Cognitive and Academic Difficulties

Very few SCD-specific interventions have been developed to address the cognitive and academic problems experienced by many children with SCD. However, researchers have started to investigate the possible cognitive and academic benefits of biomedical treatments for SCD, cognitive rehabilitation strategies, and educational interventions. Some researchers have proposed that biomedical treatments that decrease anemia severity, such as chronic transfusion therapy and oral hydroxyurea therapy, may improve cognitive functioning or prevent cognitive decline in children with SCD. These hypotheses are based on the theory that cognitive and academic problems are likely related to the impact of anemia and chronic hypoxia on the brain (Steen et al. 1999). Therefore, treatments that address these disease effects could mitigate the effect of SCD on both the brain and cognitive outcomes.

In support of this hypothesis, studies have found that both regular transfusions and oral hydroxyurea are effective in decreasing secondary stroke risk in children with a history of CVA (Adams 2000; Ware et al. 2004). Kral et al. (2003) reported that children on transfusion therapy who had elevated stroke risk, as measured by transcranial Doppler ul-trasonography, performed better on cognitive testing than children with elevated stroke risk who were not on transfusion therapy. Two recent small studies also found preliminary evidence that hydroxy-urea therapy may have cognitive benefits. A non-randomized, case-control study documented that children and adolescents taking hydroxyurea scored significantly higher on measures of verbal comprehension, fluid reasoning, and global cognitive ability than participants not taking the drug (Puffer et al. 2007). Early results of a study conducted by Thornburg et al. (2009) also showed that children taking hydroxyurea early in life do not appear to show the cognitive decline during early childhood that had previously been documented in very young children with SCD (Thompson et al. 2002). Larger studies need to be conducted to determine the cognitive effects of transfusions and hydroxyurea.

A small number of studies have examined the effects of school-based and educational interventions in children with SCD. One randomized, controlled study (Koontz et al. 2004) demonstrated that brief educational interventions with teachers (in-service instruction) and peers (classroom presentation) of children with SCD was associated with large positive effects on SCD-related knowledge in teachers, classmates, and the children with SCD themselves (Koontz et al. 2004). Children with SCD in the intervention group had significantly fewer absences than children with SCD in the control group condition, and teachers' satisfaction ratings were also higher in the intervention group. Notably, large numbers of teachers and peers in the control condition exhibited very low awareness of important risks of SCD (e.g., stroke) and misconceptions regarding the mode of SCD transmission. This finding is consistent with results showing that teachers frequently report not having adequate information about the health conditions of their students (Robinson et al. 2001). Therefore, data suggest that teachers and peers likely benefit from education related to SCD; however, research has not determined whether this type of intervention strategy can serve to improve academic performance or educational services for children with SCD. Only one small study has examined specific cognitive and academic intervention techniques for children with SCD (Yerys et al. 2003). This study, which included children with SCD and a history of cerebral infarcts, showed that children who received both tutoring and memory training showed more academic improvement than children receiving only academic tutoring (Yerys et al. 2003). Despite these promising results and the recognition that specific cognitive rehabilitation strategies are needed (King et al. 2008), no further research has been published on cognitive rehabilitation in children with SCD.

Because of the lack of specific evidence-based cognitive or academic interventions for children with SCD, most children with SCD who exhibit difficulties receive standard educational resources. In public schools in the United States, children with SCD are usually eligible for a plan that provides accommodations within the regular classroom environment (e.g., preferential seating) under the terms of Section 504 of the Rehabilitation Act of 1973 (P.L. 93-112) and the Americans With Disabilities Act of 1990 (P.L. 101-336), or an Individualized Education Program (IEP) that provides both classroom accommodations and special education services (e.g., small-group instruction, speech therapy). Children with SCD often qualify for these plans, although studies have documented that many children who would benefit from special education services do not have an IEP (Herron et al. 2003; Peterson et al. 2005). Therefore, health care teams need to understand educational services to help families obtain academic interventions. Additionally, children should receive periodic evaluations by a pediatric psychologist to assess specific cognitive and academic skills often affected by SCD and to interpret test performance with knowledge of the literature on the cognitive effects of SCD. When possible, members of the health care team should be available for school consultation, and testing reports should provide specific recommendations for school services.